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Von Willebrand disease

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https://www.readbyqxmd.com/read/29040872/highly-elevated-plasma-level-of-von-willebrand-factor-accelerates-the-formation-of-platelet-thrombus-under-high-shear-stress-in-plasma-with-deficient-adamts13-activity
#1
Hideo Yagi, Naoko Yamaguchi, Yasuaki Shida, Mitsuhiko Sugimoto, Kazuo Tubaki, Yoshihiro Fujimura, Masanori Matsumoto
Upshaw-Schulman syndrome (USS) is a thrombo-hemorrhagic disease caused by congenital deficiency of ADAMTS13 due to ADAMTS13 gene mutations. USS is characterized by repeated episodes of thrombocytopenia and microangiopathic hemolytic anemia that respond dramatically to infusions of fresh frozen plasma. There are two phenotypic expressions of USS: one is the early-onset type and the other, the late-onset type, is asymptomatic during childhood with the first bout of thrombotic thrombocytopenic purpura (TTP) developing after adolescence or during adulthood...
October 10, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/29035661/outcome-of-laparoscopic-ovariohysterectomy-or-ovariectomy-in-dogs-with-von-willebrand-disease-or-factor-vii-deficiency-20-cases-2012-2014
#2
Thomas P Keeshen, J Brad Case, Jeffrey J Runge, Ameet Singh, Philipp D Mayhew, Michele A Steffey, William T N Culp
OBJECTIVE To describe surgical techniques and perioperative management of dogs with von Willebrand disease (VWD) or factor VII (FVII) deficiency undergoing laparoscopic ovariohysterectomy or ovariectomy and evaluate outcomes. DESIGN Retrospective case series. ANIMALS 20 client-owned dogs with VWD (n = 16) or FVII deficiency (4). PROCEDURES Dogs with VWD or FVII deficiency that underwent laparoscopic ovariohysterectomy or ovariectomy between 2012 and 2014 were retrospectively identified via a multi-institutional review of medical records...
November 1, 2017: Journal of the American Veterinary Medical Association
https://www.readbyqxmd.com/read/29026409/hemostasis-in-hypothyroidism-and-autoimmune-thyroid-disorders
#3
REVIEW
Arash Ordookhani, Kenneth D Burman
CONTEXT: There are contradictory results on the effect of hypothyroidism on the changes in hemostasis. Inadequate population-based studies limited their clinical implications, mainly on the risk of venous thromboembolism (VTE). This paper reviews the studies on laboratory and population-based findings regarding hemostatic changes and risk of VTE in hypothyroidism and autoimmune thyroid disorders. EVIDENCE ACQUISITION: A comprehensive literature search was conducted employing MEDLINE database...
April 2017: International Journal of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28987857/intracranial-hemorrhage-in-patients-with-durable-mechanical-circulatory-support-devices-institutional-review-and-proposed-treatment-algorithm
#4
Wyatt L Ramey, Robyn L Basken, Christina M Walter, Zain Khalpey, G Michael Lemole, Travis M Dumont
OBJECTIVE: Spontaneous intracranial hemorrhage (ICH) is frequently managed in neurosurgery. Patients with durable mechanical circulatory support devices, including total artificial hearts (TAHs) and left ventricular assist devices (LVADs) are often encountered in the setting of ICH. While durable mechanical circulatory support devices have improved survival and quality of life for patients with advanced heart failure, ICH is one of the most feared complications following LVAD and TAH implantation...
October 4, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28987708/von-willebrand-disease-mutation-spectrum-and-associated-mutation-mechanisms
#5
REVIEW
Annika de Jong, Jeroen Eikenboom
Von Willebrand disease (VWD) is a bleeding disorder that is mainly caused by mutations in the multimeric protein von Willebrand factor (VWF). These mutations may lead to deficiencies in plasma VWF or dysfunctional VWF. VWF is a heterogeneous protein and over the past three decades, hundreds of VWF mutations have been identified. In this review we have organized all reported mutations, spanning a timeline from the late eighties until early 2017. This resulted in an overview of 750 unique mutations that are divided over the VWD types 1, 2A, 2B, 2M, 2N and 3...
September 23, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28986333/protective-effect-of-danhong-injection-combined-with-naoxintong-capsule-on-cerebral-ischemia-reperfusion-injury-in-rats
#6
Jiayang Wan, Haofang Wan, Rongbin Yang, Haitong Wan, Jiehong Yang, Yu He, Huifen Zhou
ETHNOPHARMACOLOGICAL RELEVANCE: Danhong Injection (DHI) and Naoxintong Capsule (NXT) are renowned traditional Chinese medicine in China. The drug combination of DHI and NXT is frequently applied for the treatment of cardiovascular and cerebrovascular diseases in clinic. However, there had been no pharmacological experiment studies of interaction between DHI and NXT. Due to the drug interactions, exploring their interaction profile is of great importance. MATERIAL AND METHODS: In this study, focal cerebral I/R injury in adult male Sprague-Dawley rats were induced by transient middle cerebral artery occlusion (tMCAO) for 1h followed by reperfusion...
October 3, 2017: Journal of Ethnopharmacology
https://www.readbyqxmd.com/read/28981926/-genetic-and-prenatal-diagnosis-of-a-pedigree-affected-with-type-3-von-willebrand-disease
#7
Haiyan Zhu, Chunyan Ji
OBJECTIVE: To provide genetic and prenatal analysis for a pedigree affected with type 3 von Willebrand disease. METHODS: Next generation sequencing and Sanger sequencing of the VWF gene were carried out for the pedigree. Suscepted pathogenic mutation was verified among other members of the pedigree and 100 healthy controls. Prenatal diagnosis was performed on amniotic cells derived from the fetus. RESULTS: A homozygous mutation c.7287+1G>A of the VWF gene was detected in the patient, which was predicted by bioinformatic analysis as a pathological splice site mutation...
October 10, 2017: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/28981194/elevated-preoperative-von-willebrand-factor-is-associated-with-perioperative-thrombosis-in-infants-and-neonates-with-congenital-heart-disease
#8
Ryan Hunt, Corey M Hoffman, Sirisha Emani, Cameron C Trenor, Sitaram M Emani, David Faraoni, Chava Kimchi-Sarfaty, Juan C Ibla
BACKGROUND: The surgical repair of congenital heart lesions is frequently complicated by perioperative thrombosis of unclear etiology. An imbalance between von Willebrand Factor (VWF) and ADAMTS-13 is an emerging variable in thrombosis. OBJECTIVES: To describe perioperative changes to VWF, ADAMTS-13 and NETosis and evaluate clinical and biochemical associations with postoperative thrombosis. METHODS: Neonates and infants undergoing palliation or definitive repair of congenital heart disease (CHD) were recruited (n =133)...
October 5, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28980759/comparison-of-an-automated-chemiluminescent-assay-to-a-manual-elisa-assay-for-determination-of-von-willebrand-factor-collagen-binding-activity-on-vwd-plasma-patients-previously-diagnosed-through-molecular-analysis-of-vwf
#9
E Jousselme, Y Jourdy, L Rugeri, C Négrier, C Nougier
INTRODUCTION: The correct diagnosis and classification of VWD (von Willebrand disease) is crucial and must be optimized by including the collagen binding assay (VWF:CB). VWF:CB remains an under-recognized tool, not fully automated. The objective of this study was to evaluate and to compare the previously evaluated automated chemiluminescent assay (HemosIL AcuStar VWF:CB) to the ELISA ASSERACHROM(®) assay used routinely in our laboratory in patients with molecular diagnosis of VWD. METHODS: A plasma sample from 49 patients previously diagnosed with VWD (type 1; type 2A, type 2M, type 2B) through phenotype and VWF (von Willebrand factor) analysis and 15 healthy controls was analysed...
October 5, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28974776/associations-between-snps-and-immune-related-circulating-proteins-in-schizophrenia
#10
Man K Chan, Jason D Cooper, Stefanie Heilmann-Heimbach, Josef Frank, Stephanie H Witt, Markus M Nöthen, Johann Steiner, Marcella Rietschel, Sabine Bahn
Genome-wide association studies (GWAS) and proteomic studies have provided convincing evidence implicating alterations in immune/inflammatory processes in schizophrenia. However, despite the convergence of evidence, direct links between the genetic and proteomic findings are still lacking for schizophrenia. We investigated associations between single nucleotide polymorphisms (SNPs) from the custom-made PsychArray and the expression levels of 190 multiplex immunoassay profiled serum proteins in 149 schizophrenia patients and 198 matched controls...
October 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28971901/molecular-and-clinical-profile-of-von-willebrand-disease-in-spain-pcm-evw-es-comprehensive-genetic-analysis-by-next-generation-sequencing-of-480-patients
#11
Nina Borràs, Javier Batlle, Almudena Pérez-Rodríguez, María Fernanda López-Fernández, Ángela Rodríguez-Trillo, Esther Lourés, Ana Rosa Cid, Santiago Bonanad, Noelia Cabrera, Andrés Moret, Rafael Parra, María Eva Mingot-Castellano, Ignacia Balda, Carme Altisent, Rocío Pérez-Montes, Rosa María Fisac, Gemma Iruín, Sonia Herrero, Inmaculada Soto, Beatriz de Rueda, Victor Jimimenez-Yuste, Nieves Alonso, Dolores Vilariño, Olga Arija, Rosa Campos, María José Paloma, Nuria Bermejo, Rubén Berrueco, José Mateo, Karmele Arribalzaga, Pascual Marco, Ángeles Palomo, Lizheidy Sarmiento, Belén Iñigo, María Del Mar Nieto, Rosa Vidal, María Paz Martínez, Reyes Aguinaco, Jesús María César, María Ferreiro, Javier García-Frade, Ana María Rodríguez-Huerta, Jorge Cuesta, Ramón Rodríguez-González, Faustino García-Candel, Rosa Cornudella, Carlos Aguilar, Francisco Vidal, Irene Corrales
Molecular diagnosis of patients with von Willebrand disease is pending in most populations due to the complexity and high cost of conventional molecular analyses. The need for molecular and clinical characterization of von Willebrand disease in Spain prompted the creation of a multicenter project (PCM-EVW-ES) that resulted in the largest prospective cohort study of patients with all types of von Willebrand disease. Molecular analysis of relevant regions of the VWF, including intronic and promoter regions, was achieved in the 556 individuals recruited by development of a simple, innovative, relatively low-cost protocol based on microfluidic technology and next-generation sequencing...
September 29, 2017: Haematologica
https://www.readbyqxmd.com/read/28957942/purification-and-characterization-of-a-new-highly-pure-double-virus-inactivated-von-willebrand-factor-concentrate
#12
Filippo Mori, Marcella Maddaluno, Claudio Farina
: Desmopressin-unresponsive von Willebrand disease patients are treated with substitutive therapy, with both pure von Willebrand factor (vWF) and factor VIII/vWF concentrates. We developed a new purification process, easily scalable to industrial level, to obtain a double virus inactivated highly pure vWF. VWF was purified starting from a waste fraction of already in use human plasma-derived factor VIII manufacturing procedure, using only one anionic-exchange chromatographic step. After chromatography, the product was dialyzed, lyophilized, and heat treated...
September 27, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28940121/anti-cenp-b-and-anti-topo-1-containing-sera-from-systemic-sclerosis-related-diseases-with-raynaud-s-phenomenon-induce-vascular-endothelial-cell-senescence-not-via-classical-p53-p21-pathway
#13
Chieh-Yu Shen, Ko-Jen Li, Pei-Hsuan Lai, Chia-Li Yu, Song-Chou Hsieh
Raynaud's phenomenon (RP) is the earliest and most common clinical manifestation in patients with systemic sclerosis (SSc) and its related diseases containing anti-TOPO-1 and/or anti-CENP-B autoantibodies in the sera. However, the cause-effect relationship between the two autoantibodies and RP remains elucidation. Sera containing anti-CENP-B and anti-TOPO-1 autoantibodies were obtained from SSc-related diseases manifesting RP. The polyclonal auto-antibodies were purified from pooled sera by affinity chromatography...
September 23, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28927148/association-of-abo-blood-groups-with-von-willebrand-factor-factor-viii-and-adamts-13-in-patients-with-lung-cancer
#14
Xia Liu, Xiaogang Chen, Jiezuan Yang, Renyong Guo
Coagulative and fibrinolytic disorders appear to be associated with the development of lung cancer. The aim of the present study was to determine plasma levels of von Willebrand factor (VWF) and a disintegrin and metalloproteinase with a thrombospondin type 1 motif 13 (ADAMTS-13), and factor VIII (FVIII) activity, in association with O and non-O blood groups in patients with lung cancer. Plasma levels of VWF and ADAMTS-13, and FVIII activity were measured in 115 patients with lung cancer and 98 healthy subjects...
September 2017: Oncology Letters
https://www.readbyqxmd.com/read/28926986/efficacy-of-octocog-alfa-advate-in-a-child-with-type-3-von-willebrand-disease-and-alloantibodies
#15
Gianluca Sottilotta, Francesca Luise, Elisabetta Massara, Vincenzo Oriana, Angela Piromalli
Von Willebrand disease (VWD) is the most frequent inherited bleeding disorder and is caused by either a quantitative and/or qualitative defect of the multimeric glycoprotein vonWillebrand factor (VWF).[...].
September 18, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28915230/albuminuria-and-endothelial-dysfunction-in-patients-with-non-diabetic-chronic-kidney-disease
#16
Meng-Jie Huang, Ri-Bao Wei, Jing Zhao, Ting-Yu Su, Qing-Ping Li, Xi Yang, Xiang-Mei Chen
BACKGROUND Albuminuria has been associated with cardiovascular events, but whether such an association can be explained by endothelial dysfunction is not fully understood. In this study, we examined the relationship between the urine albumin-to-creatinine ratio (UACR) and biomarkers of endothelial function in patients with chronic kidney disease (CKD). MATERIAL AND METHODS The cross-sectional associations of renal dysfunction and UACR with procoagulant and inflammatory factors were evaluated for 151 consecutive CKD (stage 3-5) patients...
September 15, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28904675/von-willebrand-s-disease-case-report-and-review-of-literature
#17
REVIEW
Hanae Echahdi, Brahim El Hasbaoui, Mohamed El Khorassani, Aomar Agadr, Mohamed Khattab
Von Willebrand Disease (VWD) is the most common human inherited bleeding disorder due to a defect of Von Willebrand Factor (VWF), which a glycoprotein crucial for platelet adhesion to the subendothelium after vascular injury. VWD include quantitative defects of VWF, either partial (type 1 with VWF levels < 50 IU/dL) or virtually total (type 3 with undetectable VWF levels) and also qualitative defects of VWF (type 2 variants with discrepant antigenic and functional VWF levels). The most bleeding forms of VWD usually do not concern type 1 patients with the mildest VWF defects (VWF levels between 30 and 50IU/dL)...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28885052/migraine-and-vascular-disease-biomarkers-a-population-based-case-control-study
#18
Gretchen E Tietjen, Jagdish Khubchandani, Nabeel Herial, Inge H Palm-Meinders, Hille Koppen, Gisela M Terwindt, Mark A van Buchem, Lenore J Launer, Michel D Ferrari, Mark C Kruit
Background The underpinnings of the migraine-stroke association remain uncertain, but endothelial activation is a potential mechanism. We evaluated the association of migraine and vascular disease biomarkers in a community-based population. Methods Participants (300 women, 117 men) were recruited as a part of the Dutch CAMERA 1 (Cerebral Abnormalities in Migraine, an Epidemiologic Risk Analysis) study. Participants were aged 30-60 (mean 48) years, 155 migraine had with aura (MA), 128 migraine without aura (MO), and 134 were controls with no severe headaches...
January 1, 2017: Cephalalgia: An International Journal of Headache
https://www.readbyqxmd.com/read/28874064/the-association-of-aging-with-von-willebrand-factor-levels-and-bleeding-risk-in-type-1-von-willebrand-disease
#19
Craig D Seaman, Margaret V Ragni
Little is known about aging in von Willebrand disease (VWD). It is uncertain whether VWD patients experience an age-related increase in von Willebrand factor (VWF) levels, and if so, it is unknown whether normalization of VWF levels with aging ameliorates bleeding risk. We aimed to determine the association of age with VWF levels and bleeding risk in patients with type 1 VWD. This is a retrospective chart review of patients with type 1 VWD presenting to the Hemophilia Clinic of Western Pennsylvania for regularly scheduled clinic visits...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28866059/revascularization-operation-for-moyamoya-disease-with-concurrent-von-willebrand-disease
#20
Kenji Miki, Koichi Arimura, Ataru Nishimura, Koji Yoshimoto, Tetsuro Sayama, Koji Iihara
BACKGROUND: Although extracranial-intracranial (EC-IC) bypass is an effective treatment strategy for symptomatic moyamoya disease, surgeons need to be cautious regarding the possibility of postoperative hemorrhagic complications in patients with a concurrent coagulation disorder. Here, we describe a case of EC-IC bypass for moyamoya disease concurrent with von Willebrand disease type 1. CASE DESCRIPTION: Following perioperative replacement of the von Willebrand factor, the patient showed an uneventful and uncomplicated clinical course...
September 1, 2017: World Neurosurgery
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